Treatment of pemphigus vegetans to rituximab refractory to conventional therapy.

An otherwise healthy 53-year-old woman was admitted to our Dermatologic Clinic for evaluation of two erythematous plaques on the front and back of both her thighs, which had developed over the previous ten months (Figure 1A). These plaques showed vegetative or hypertrophic elements, and were occasionally pruritic. The rest of her skin and mucosal surfaces were normal, the patient being in good health. A complete workup was carried out, including a complete blood count and biochemical profile, C-reactive protein count, erythrocyte sedimentation rate, chest X-ray, purified protein derivative (PPD) test and human immunodeficiency virus (HIV) serology, which showed no abnormalities. Bacterial and fungal cultures were negative as well. Histopathological evaluation of a skin specimen revealed acanthosis and papillomatosis in the epidermis with predominantly eosinophilic microabscesses. Direct immunofluorescence assay showed deposits of immunoglobulin G (IgG) and C3 in the lower part of the epidermis, which was consistent with pemphigus. Thus, based on clinical and pathological findings, the patient was diagnosed with pemphigus vegetans (PV). Treatment with clobetasol propionate applied in occlusive cures proved ineffective after 2 months. The patient was then placed on oral prednisone at a dose of 1 mg/kg/day for 30 days with posterior tapering. However, in addition to her irresponsiveness to